Likely treatment

Clotting factor replacement is only required after significant injury or trauma. It is not required frequently enough to require home supplies and self treatment.

Mobility

Mobility will depend on whether there is joint damage to the lower limbs. The probability of this will depend on the customer’s age. A small proportion of people with mild haemophilia born before 1970 have significant joint damage from untreated bleeds that occurred before clotting factor treatment was available. Joint damage is not likely to be widespread and in this group joint replacement, particularly knee replacement can improve mobility. This is because in many cases most of the other joints are healthy, having not been affected by bleeding episodes.

Care

Personal care may be difficult if the elbow or shoulder is significantly affected and range of movement in both these joints is poor. This is rare and is only likely in adults born before 1970. In these cases, help may be required with personal hygiene, dressing, meal preparation and administration of treatment.

Supervision by others does not prevent bleeds or prevent adults with normal cognitive function from sustaining injuries. Adults are able to recognise the onset of bleeding and seek help or self-treat.

Likely treatment

Likely to self administer clotting factors on an as required basis.

Mobility

Mobility will depend on whether there is joint damage to the lower limbs. The probability of this will depend on their age. People born before 1970 will have significant joint damage from untreated bleeds that occurred before clotting factor treatment was available. People born from 1970 onwards may have some joint damage depending on their frequency of their bleeding and the number of significant bleeds into joints they have had over the years.

If mobility is restricted, this will be because of pain related to arthritis in the hips, knees and ankles. Joint replacement of individual joints will relieve pain from arthritis and prevent further bleeding into that joint but will not improve range of movement or mobility. This is because other joints are affected. If a ‘fixed flexion’ deformity of the knee or hip is present then mobility is especially likely to be reduced, a flexion deformity effectively shortens the affected leg and affects gait. In someone with multiple damaged joints, this places further strain on other joints and increases the risk of bleeding when walking.

People with moderate haemophilia born more recently are likely to have very little joint damage. This is because clotting factor treatment can be administered at the onset of bleeding at home before a significant amount of blood has collected in the joint.

Care

Personal care may be difficult if the elbow or shoulder is significantly affected and range of movement in both these joints is poor. This is much less common than mobility problems due to lower limb involvement. It is particularly likely in adults born before 1970.

Supervision by others does not prevent bleeds or prevent adults with normal cognitive function from sustaining injuries. Adults are able to recognise the onset of bleeding and seek help or self-treat.

Likely treatment

Likely to be on self administered prophylactic (preventative) clotting factor treatment at home. This involves several injections a week as described under treatment. The majority of adults are able to administer this themselves. Bleeding episodes are recognised early and extra clotting factors administered as described so preventing further joint damage form uncontrolled bleeding.

Mobility

Mobility will depend on whether there is joint damage to the lower limbs. The probability of this will depend on their age. People born before 1970 are likely to have significant joint damage from untreated bleeds that occurred before clotting factor treatment was available. People born from 1990s onwards may have no or minimal joint damage as large bleeds into joints have been effectively prevented by prophylactic treatment.

People born after 1986 may also have received prophylactic (preventative) treatment and be mobile. The majority will have some joint damage. Mobility is likely to be restricted because of pain on walking related to arthritis in the hips, knees and ankles. Joint replacement of individual joints will relieve pain from arthritis and prevent further bleeding into that joint but will not improve mobility or range of movement. This is because other joints are affected and they will still be painful on walking e.g. ankle pain becomes more noticeable after knee replacement. If flexion deformity of the knee or hip is present than mobility is especially likely to be reduced, a flexion deformity effectively shortens the affected leg and affects gait. In someone with multiple damaged joints this places further strain on other joints and increases the risk of bleeding when walking. People born before 1980 are likely to have multiple affected joints and mobility problems.

Care

Personal care may be difficult if the elbow or shoulder is significantly affected and range of movement in both these joints is poor. This is much less common than mobility problems due to lower limb involvement. It is particularly likely in adults born before 1970. In these cases help may be required with personal hygiene, dressing, meal preparation and administration of treatment.

Supervision by others does not prevent bleeds or prevent adults with normal cognitive function from sustaining injuries. Adults are able to recognise the onset of bleeding and seek help or self-treat.